Researchers in the United States say lung transplants do little to improve the outcomes for children with severe cystic fibrosis (CF) and often increases their risk of dying.
It seems despite costly and complex surgery only 5 of the 514 American children, age 18 and younger, on the waiting list for a transplant from 1992 through 2002 lived any longer as a result of the operation.
Of the 248 children who actually received new lungs in the 11-year period, only one patient showed a clear benefit from transplantation, while 162 (almost two-thirds), were at a higher risk of dying after the procedure.
The researchers were unable to determine the harm or benefit of transplantation in the remaining 85 children who received new lungs.
The revelation will be a blow to the many young sufferers of the disease and their families as lung transplantation is an accepted treatment for severe cases of the fatal genetic disease.
Dr. Theodore Liou and his team at the University of Utah in Salt Lake City say they were shocked at the finding.
They embarked on the study believing there would be a number of patients who would benefit along with some who would not, but it did not balance out.
Cystic fibrosis affects around 70,000 people worldwide; it is a hereditary disease that attacks multiple systems in the body, most dramatically affecting the lungs and gastrointestinal tract.
The disease creates an abnormally thick mucus that clogs the lungs and digestive system.
Relatively few studies have examined the effectiveness of lung transplants for helping treat the incurable disease but this study is the most extensive study of the issue to date.
Although the Cystic Fibrosis Foundation says that 50 percent of recipients are alive five years after surgery, complications from transplants are responsible for 12 percent of all deaths among people with CF.
There have also been suspicions that the operation may not be best for sufferers under 18 as the five-year survival rate of 33 percent for children is less than for adults or people who have received a transplant for another reason.
Dr. Liou says the implication is that most children with CF are going to be harmed by lung transplantation and there should not be the expectation that lung transplantation will prolong their lives.
Dr. Liou says the study emphasises the need for CF patients to follow a consistent regimen of conventional therapy and medical care to maximize their survival.